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Introduction of "vario plates" for retention after mandibular distraction osteogenesis.

This report introduces a new removable orthodontic appliance called "vario plates" for retention following distraction osteogenesis of the mandible. The "vario plates" consist of removable orthodontic appliances in the maxilla and the mandible. These are fabricated out of self-curing resin with typical wire elements. They are connected with telescoping maxillomandibular guidance rods, which have a smoothly variable length, from the maxillary molar region to the mandibular premolar region on each side. The telescope on both sides is adjustable in this length by means of a protrusion nut. Thus, it is possible to move the mandible forward an exactly controlled amount. The "vario plates" are in function for 24 hours a day in the patient for the first 6 months after mandibular distraction osteogenesis and subsequently only at night. The application of the plates is demonstrated in a patient with Goldenhar syndrome. Application of "vario plates" after distraction osteogenesis makes it possible to hold the mandible in a stable position. The combination of maxillofacial surgery with distraction osteogenesis and orthodontic treatment and retention leads to an improvement in therapy of patients with severe dentofacial anomalies.

Adult↗

Expression of cyclooxygenase genes and involvement of endogenous prostaglandin during osteogenesis in the rat tibial bone marrow cavity.

Many researchers have demonstrated that the administration of prostaglandins (PGs), especially PGE2, increases the bone volume in vivo. It is still not clear how endogenous PG is associated with such bone formation. Cyclooxygenase (COX), which acts in the synthesis of PG from arachidonic acid, has been recently revealed to have two subtypes, a constitutive type (COX-1) and an inducible one (COX-2). We examined the expression of each COX subtype during osteogenesis with the established model of the rat tibial medullary cavity in which osteogenesis can be induced by the injection of colchicine. The expression of both COX-1 and COX-2 genes was enhanced after colchicine injection in the early stage before the start of bone formation. Only the COX-2 gene expression was elevated again later during the beginning of bone formation. Furthermore, the daily administration of indomethacin, COX inhibitor, could reduce the bone mass induced by colchicine in the rat tibiae. These data indicate that endogenous PGs are associated with osteogenesis in this model. Moreover, the present study suggests that COX-2 and COX-1 would both be involved in the early stage of osteogenesis, and COX-2 is likely to be more associated with the maturation of osteoblasts in the later stage.

Animals↗

[Valvular heart surgery in osteogenesis imperfecta].

Osteogenesis imperfecta is a disease in which fragile bones readily cause fracture. Valvular disease concurrently develops. However, the surgery-related mortality rate is approximately 30%. In this study, we report 2 patients with osteogenesis imperfecta who underwent valvular heart surgery. Patient 1 was a 31-year-old male. He had previously been diagnosed as having osteogenesis imperfecta. Echocardiography suggested aortic valve insufficiency, and aortic valve replacement was performed. Patient 2 was a 59-year-old male. During admission, osteogenesis imperfecta was diagnosed. Echocardiography suggested mitral valve insufficiency, and mitral valve plasty was performed. In the 2 patients, intraoperative hemorrhage was marked. However, there were no fatal complications. We also reviewed the literature.

Adult↗

[The influence of azathioprine on the osteogenesis of the limbs].

BACKGROUND: The purpose of this experimental work was to examine the osteogenesis of rats limbs under the influence of immunosuppressant azathioprine. MATERIAL AND METHODS: Two doses of azathioprine (50mg/kg and 80 mg/kg) were administered intraperitoneally to experimental and control pregnant rats once during the period from the 1(st) to the 14(th) day of embryogenesis. The size of the centers of ossification was measured in the limbs fixed in ethanol, cleared and stained with alizarin red. RESULTS: The analysis of the experimental data revealed that osteogenesis was sensitive to the influence of azathioprine administered to embryos from the 4(th) to the 13(th) day of embryogenesis. The centers of ossification in embryos treated with azathioprine on the 10 and 12(th) days were most damaged: shorter, deformed or absent. CONCLUSIONS: The limb osteogenesis was delayed and damaged by azathioprin in the period of active organogenesis. The bigger the doze of azathioprine the more osteogenesis was delayed. The most sensitive was the skeleton of the autopod.

Animals↗

A case of cerebellar hypoplasia in a Chinese infant with osteogenesis imperfecta.

We report a unique case of unilateral cerebellar hypoplasia in a young Chinese girl with osteogenesis imperfecta type IV. Magnetic resonance imaging showed mild basilar invagination and impression. Although unilateral cerebellar hypoplasia and osteogenesis imperfecta may have been coincidental diagnoses, we propose possible mechanisms for unilateral cerebellar hypoplasia secondary to osteogenesis imperfecta. For example, cerebellar hypoplasia may have been because of vascular disruption or direct compression to the posterior circulation in utero. Foetuses with osteogenesis imperfecta are more susceptible to the above risks compared to the normal foetus because of associated craniocervical anomalies and a poorly ossified skull.

Asian People↗

Alveolar distraction osteogenesis for the correction of vertically deficient edentulous ridges: a multicenter prospective study on humans.

PURPOSE: The purposes of this prospective multicenter study were to evaluate the use of vertical distraction osteogenesis in the correction of vertically deficient alveolar ridges and to evaluate whether the vertical bone gained by distraction osteogenesis was maintained over time when dental implants were placed in the distracted areas. MATERIALS AND METHODS: Thirty-seven patients presenting vertically deficient edentulous ridges were treated in 4 different centers by means of distraction osteogenesis with an intraoral alveolar distractor. Two to 3 months after consolidation of the distracted segments, 138 dental implants were placed in the distracted areas. Four to 6 months later, abutments were connected and prosthetic loading of the implants began. RESULTS: The mean follow-up after initial prosthetic loading was 34 months (range 15 to 55 months). The mean bone gain obtained by distraction was 9.9 mm (range 4 to 15 mm). The cumulative success rate of the implants 4 years after the onset of prosthetic loading was 94.2%, while the implants' cumulative survival rate was 100%. No statistically significant differences were found between the different centers as far as survival and success rates of implants were concerned. DISCUSSION AND CONCLUSION: The results of this study appear to demonstrate that distraction osteogenesis is a reliable technique for the correction of vertically deficient edentulous ridges. The regenerated bone appeared to withstand the functional demands of implant loading. The survival and success rates of the implants placed in the distracted areas were consistent with those reported in the literature regarding implants placed in native bone in this patient population.

Adolescent↗

[Distraction osteogenesis in patients with syndromic midface retrusion--a primary experience].

OBJECTIVE: To study the feasibility of midface distraction for correction of severe syndromic Four consecutive patients with severe syndromic midface retrusion underwent midface retrusion. METHODS: distraction osteogenesis. The patients(three girls and one boy) aged from 4 to 12 years. Two were with Crouzon syndrome, one with Apert and one with Marfan syndrome. One was treated with Le Fort III external distraction, two with Le Fort III internal distraction, and the other with monobloc internal distraction. The distraction devices were activated on the fourth postoperative day at 1 mm per day. RESULTS: All patients completed the distraction as activated on the fourth postoperative day at 1 mm per day. Results was planned. Successful advancement of 8 to 20 mm was obtained at the occlusal level in all patients as measured by cephalograms. The facial appearance was significantly improved,especially in the orbits and the upper part of the nose. Follow-up from 4 months to one year demonstrated that the face was symmetrical. All patients obtained This study shows that although midface distraction osteogenesis needs to be satisfactory results. CONCLUSIONS: This study shows that although midface distraction osteogenesis needs to be improved to increase its controllability, it has obvious advantages over the traditional way of bone graft and rigid fixation. Midface distraction avoids bone grafts and alleviates the restriction of the soft tissue to midfacial bone advancement. Midface distraction osteogenesis is an effective and practical way to correct severe syndromic midfacial hypoplasia.

Australia↗

[Mandibular distraction osteogenesis: an experimental study in goats].

OBJECTIVE: This experiment was designed to develop an experimental model of mandibular distraction osteogenesis in goats for determining the principle and the process of distraction osteogenesis. METHODS: Eight 3-month-old, healthy domestic goats were randomly divided into four groups (n = 2 per group). The animals from each group were killed at 1, 2, 4, and 6 weeks after bone distraction, respectively. The sections of newly formed bone were obtained and evaluated with histologic and radiographic study. We also assessed the newly formed bone with bone density examination. RESULTS: The specimens of right mandible increased averagely 10 mm after distraction. The Newly formed bone was demonstrated histologically. The X-ray examination and bone density measurements showed that the bone density increased with the time of fixation. CONCLUSION: Goat is a proper experimental animal for modeling mandibular distraction osteogenesis, and in this regal, it is important to keep the direction of distracter force in conformity with the direction of the expected distraction osteogenesis for avoiding the side force and thus ensuring successful distraction and good quality of newly formed bone.

Animals↗

[Orthognathic surgery and distraction osteogenesis for treatment of obstructive sleep apnea hypopnea syndrome].

OBJECTIVE: To discuss the indication and protocol of surgical therapy when treating mandibular micrognathism accompanying obstructive sleep apnea-hypopnea syndrome (OSAHS) using distraction osteogenesis and orthognathic surgery. METHODS: A total of 17 patients with mandibular micrognathism accompanying moderate to severe OSAHS, aged 11 to 59 years, 15 men and 2 women, were reviewed. Four of them were treated with orthognathic surgery, 5 of them were treated with distraction osteogenesis, and the other 8 patients were treated with an integrated procedure combining distraction osteogenesis with orthognathic surgery. Cephalometric analysis and polysomnography studies were obtained pre- and postoperatively. RESULTS: SNB angle changed from 64.6 degrees to 71.9 degrees, post airway space (PAS) from 5.4mm to 13.2 mm, apnea and hypopnea index (AHI) from 58.4 to 7.6, lowest saturation of oxygen (LSAT) from 66% to 87%. All the differences showed statistical significance (P < 0.001). The average mental horizontal advancement (MHA) was 14.3 mm and its correlation coefficients with deltaSNB, deltaPAS, deltaAHI, and deltaLSAT were 0.36, 0.62, 0.34, and -0.14, respectively. CONCLUSIONS: Both distraction osteogenesis and orthognathic surgery can be effectively used to treat patients with mandibular micrognathism accompanying OSAHS with slightly different indications. A combination of these two operations may be preferred.

Adolescent↗

[Influence of bisphosphonate treatment on the radiological features of osteogenesis imperfecta in children].

The Osteogenesis imperfecta (OI) is a hereditary disturbance of collagen biosynthesis. The illness is characterized by frequent fractures that consequently cause functional disturbance of locomotion system as well as deformation of long tubular bones. The purpose of the present work was to study radiological features of clinical forms of osteogenesis imperfecta during bisphosphonate treatment in children according to the schemes we developed taking into consideration the type of illness and the age of the patient. The total number of 32 patients, aged 1-15, suffering from osteogenesis imperfecta, have been investigated. The patients have been divided into three groups depending on the type of illness (I, III and IV type). Type II (lethal form) has not been observed by us. All patients, participating in the study underwent radiological examination of tubular bones and vertebra pre and post treatment. The results of the study showed that bisphosphonate treatment has positive effects in patients of type I and IV of osteogenesis imperfecta and is less effective in the type III of the diseases.

Adolescent↗

[Study on the osteogenesis ability of co-culturing bone marrow stromal cells (BMSCs) and small intestinal submucosa].

PURPOSE: Using co-culturing of bone marrow stromal cells (BMSCs) and small intestinal submucosa (SIS), to evaluate the cellular compatibility and the osteogenesis ability as well as the feasibility of SIS to serve as a scaffold in tissue engineering. METHODS: Bone marrow was aspirated from the healthy adult goat and culture-expanded in vitro by the method of total marrow. The third passage cells were transplanted into SIS after being induced to differentiate into osteoblasts for 4 days and then seeded into SIS for 3 to 10 days. BMSCs alone were cultured at the same condition to act as controls. The cellular morphology and function (attachment, proliferation and differentiation) and the ectopia osteogenesis in vivo were assessed separately by means of phase contrast microscope, SEM and MTT, ALP activity, histology detection. The difference between the two groups were analyzed by Student's t test statistically. RESULTS: The BMSCs could adhere to SIS and proliferate and grow on the surface of SIS normally. The cellular activity and function were not affected by SIS, no statistical difference was found between the two groups (P>0.05). The BMSCs/SIS compounds had good abilities of ectopia osteogenesis in vivo, and with time going on new bone formation increased. There was no bone formation in the control group. The endochondral bone formatin was the main pattern of osteogenesis. CONCLUSIONS: As a natural, absorbable, acellular scaffold, SIS is biocompatible and can be used as a tissue engineering scaffold. And for a membrane feature, there is the possibility to construct tissue engineered bone membrane with SIS.

Adult↗

[Hearing loss in osteogenesis imperfecta--casuistic demonstration].

INTRODUCTION: Osteogenesis imperfecta (OI) is a genetic disorders of connective tissue. Hearing loss has been repeatedly reported as a major syndrome of osteogenesis imperfecta. The hearing loss in OI is predominantly of the conductive type. MATERIAL AND METHODS: The family (mother and two daughters) suffering from osteogenesis imperfecta are presented. RESULTS: Audiological examination is presented. It revealed various types of hearing loss in members of family (conductive, sensorineural, mixed). CONCLUSIONS: On this example variety of hearing problems in this disease are demonstrated. Etiology and classification of osteogenesis imperfecta, clinical proceeding and treatment are presented.

Adolescent↗

[Experimental studies on ectopic osteogenesis by seeding human mesenchymal stem cell on human natural bone derived scaffold materials].

OBJECTIVE: To investigate the ectopic osteogenesis potential of human natural bone derived material combined with human bone marrow mesenchymal stem cells (MSCs). METHODS: Cell-scaffold complexes were implanted subcutaneously into the left back of the nude mice, and human natural bone derived material were implanted into the right back as control group. The mice were killed respectively on the postoperative 2 weeks, 4 weeks and 8 weeks. The macroscopic, histopathological, alkaline phosphatase (ALP) activity assay methods were performed to assess the ectopic osteogenesis potential. RESULTS: The cartilaginous osteogenesis were observed in both deproteinated bone and decalcified bone, and the more new bone tissue formed gradually as the time went by after implantation. ALP activity become stronger followed with the time (P < 0.05), and compared with the decalcified bone, deproteinated bone displayed stronger ALP activity (P < 0.05). CONCLUSION: The MSCs and human natural bone derived material can be used as good seed cells and scaffold materials respectively to construct tissue-engineered bone, and as the scaffold material, deproteinated bone has better osteogenesis ability than decalcifed bone.

Animals↗

RNA sequence analysis of a perinatal lethal osteogenesis imperfecta mutation.

The perinatal lethal form of osteogenesis imperfecta often results from mutations which disrupt stable assembly, delay secretion, and cause excessive posttranslational modification of type I procollagen molecules. One such mutation was efficiently characterized by an indirect method of RNA sequence analysis. The mutation initially was localized in procollagen by mapping the distribution of abnormal posttranslational modification within the triple helical domain of mutant molecules. Total RNA was isolated from osteogenesis imperfecta cells in culture, cDNA was synthesized using alpha 1(I) and alpha 2(I) specific primers, and fragments of cDNA suspected to harbor the mutation were amplified by the polymerase chain reaction technique and then cloned in M13 vectors. Sequence analysis of the amplified cDNA revealed a new, heterozygous Gly----Val substitution at residue 256 of the triple helical domain of alpha 1(I) chains produced by the perinatal lethal osteogenesis imperfecta cells. The nature and location of the mutation were confirmed by sequence analysis of amplified genomic DNA. A Gly----Val substitution has not previously been associated with the lethal form of osteogenesis imperfecta, and this mutation has the most amino-terminal location within the alpha 1(I) chain triple helical domain reported to date.

Alleles↗

Operative treatment of spine deformity in osteogenesis imperfecta.

There has been little mention in the literature of scoliosis associated with osteogenesis imperfecta. The purpose of this study was to investigate the problem of spine deformity in a series of osteogenesis imperfecta patients and to review the operative treatment of 8 patients. A series of 49 patients (ranging in age from 8 months to 57 years, average 12 years) with osteogenesis imperfecta were studied. Thirty-five (71%) of these patients had scoliosis ranging from 7 degrees to 105 degrees (average 42 degrees). The more severely involved patients (OIC and OITI groups) demenostrated a higher incidence (80%) of scoliosis and a greater average degree of curvature (42 degrees) as compared to the mildly involved (OITII) group with a 50% incidence of scoliosis with an average curvature of 26 degrees. Attempts to control these curves by bracing have been difficult and usually had to be abandoned. Eight of these patients underwent spine stabilization in an attempt to control curve progression. All 8 patients had posterior spine fusions. One of these patients had an anterior fusion preceding the posterior fusion because of a significant kyphotic deformity. Harrington instrumentation was used in 5 patients. One patient could not be instrumented secondary to severe osteoporosis while the remaining 2 patients were treated by the Risser technique. Postoperatively, either cast or brace protection was used. Only minimal improvement in the curves was achieved. Spine stabilization rather than curve correction is the goal of spine fusion in osteogenesis imperfecta. An important finding was that there was no change in the pre- and postoperative activity and ambulatory status in these patients. Preliminary follow-up (9-12 months) in all 8

Adolescent↗

Upper limb surgery in osteogenesis imperfecta.

Although 90% of the surgical procedures performed on patients with OI are on the lower limbs, intramedullary fixation of the humerus may at times be indicated also in the upper limb for the correction of deformity and improvement of function. Upper limb deformities are generally present only in patients with severe disease (osteogenesis imperfecta congenita or osteogenesis imperfecta tarda I). The recommendations expressed in this paper are based on experience with 36 intramedullary rod fixation procedures on the upper limb in 12 patients with osteogenesis imperfecta, including 24 on the humerus, five on the radius and seven on the ulna. A Rush pin provided satisfactory fixation for the humerus, when inserted from its proximal end. Intramedullary rods were extremely difficult to insert in the forearm bones, and the author considers the operation to be rarely indicated. Two interesting and unusual problems in the upper extremities have been encountered in patients with osteogenesis imperfecta: deformity of the clavicle severe enough to cause pain and limited motion, and unicameral bone cysts in the upper humerus.

Adolescent↗

[The radiotherapy of hyperplastic heterotopic ossification in osteogenesis imperfecta. 2 case reports].

PURPOSE: Osteogenesis imperfecta is a rare hereditary disease of connective tissue with a genetic defect in collagen synthesis. In osteogenesis imperfecta hyperplastic heterotopic ossification can be induced by hyperplastic callus formation caused by trauma or operation. Heterotopic ossifications can be found in numerous benign diseases. The successful use of low dose radiotherapy in the treatment of heterotopic ossifications is well-known from the literature. PATIENTS AND METHODS: We treated two children (a 13-year-old girl and a ten-year-old boy) with heterotopic ossifications of the lower extremities in osteogenesis imperfecta type IV (Lobstein) with a low dose irradiation (10 x 1 Gy, respectively 6 x 1 Gy) under megavoltage conditions. RESULTS: After radiotherapy the children were painfree and the hyperplastic callus was considerably reduced. The previously immobilized patients could partly be mobilized. Thereby it could be contributed to the rehabilitation of the patients. New hyperplastic callus formation was not observed in the irradiated areas so far. CONCLUSION: Analogous to the successful radiation of heterotopic ossifications in other benign diseases radiation therapy seems to be a successful treatment of hyperplastic callus formation in osteogenesis imperfecta. Despite the late risks of radiotherapy radiation treatment of benign diseases in children might be indicated.

Adolescent↗

[Osteogenesis imperfecta with hypertrophic callus. Apropos of 2 cases with early onset].

BACKGROUND: The callus that forms at the site of recent fractures in patients with osteogenesis imperfecta is usually normal, but hyperplastic callus may develop. CASE REPORTS: Case 1. A hyperplastic callus with local inflammation developed at the site of a fracture of the left thigh of a boy, aged 11 months suffering from osteogenesis imperfecta. This disease was characterized by the progressive development of broad limbs, bone islands on skull x-rays and blue sclerae. He had also had fractures at the ages of 4 and 7 months. Follow-up showed that this boy, now aged 15 years, had several fractures with the development of similar hyperplastic calluses. These limitated joint motility, causing him to remain confined to his bed. Case 2. This girl of consanguineous parents had a fracture of the femora at the age of 1 month; she had blue sclerae and developed a hyperplastic callus. The roentgenographic studies showed generalized osteopenia and deformities indicating osteogenesis imperfecta. CONCLUSION: Hyperplastic callus may develop in osteogenesis imperfecta. Its diagnosis may be difficult with neoplasms of bone, such as osteosarcoma, and its treatment is difficult.

Bony Callus↗